Huntington’s Gene-inducing Damage Might Have a Control Mechanism

Texas Tech University Health Sciences Center (TTUHSC) researchers have identified a molecule — mitochondrial division inhibitor 1 (Mdivi1) — believed to hold promise as a potential treatment for Huntington’s disease. Mitochondria are specialized organelles in a cell responsible for creating the energy needed by the body to sustain life.

An estimated 30,000 Americans have Huntington’s disease (HD), but another 200,000 are at risk of falling victim, because every child of a parent with HD has a 50 percent chance of inheriting the disease. Huntington’s, marked by the progressive destruction of striatal neurons in the brain, affects cognitive processes and memory, and its symptoms include uncontrolled, rapid and jerky movements called chorea (HD used to be known as “Huntington’s chorea”), and athetosis — a condition characterized by relatively slow involuntary movements.

According to the research, mitochondrial division inhibitor 1 is capable of reducing excessive mitochondrial fragmentation induced by mutant huntingtin ( a protein that expresses expanded polyglutamine repeats in cells of patients with Huntington’s disease), while maintaining mitochondrial function and improving synaptic activity in Huntington’s disease neurons.

“When the mitochondria are compromised in neurons from brain disease such as Huntington’s, injury and even death to the cell occurs,” Dr. Reddy said in a TTUHSC release. “Mitochondrial damage and synaptic dysfunction are the prominent features in neurons from patients with Huntington’s disease. These findings suggest that mitochondrial division inhibitor 1 will improve mitochondrial function and protect against synaptic damage in Huntington’s disease. This may be a promising molecule for treatment for the Huntington’s disease patient.”

It is also suggested that this study’s findings may have therapeutic implications for all brain diseases in which expanded polyglutamine repeats as genetic mutations.

Huntington’s disease is currently incurable, and patients require full-time care at the disease’s end stages. Several pharmaceutical and non-drug treatments can relieve some HD symptoms, but have only limited efficacy in improving quality of life.

Charles Moore has a long-proven track record in both print and digital journalism, touching on a wide range of subjects, from biotech and healthcare to politics and technology. He contributes substantial feature articles research and development for the news site, particularly pertaining to technology.

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